Aust -
N Z J Med (1979). 9, pp 568 570
CASE REPORT
Systemic Lupus Erythematosus Presenting as Post-partum Chorea D. Thomas', P. D. Byrnet and R.
L. Travers:
From the Mayday Hospital, Thornton Heath, London, UK
SUtnmary: Systemic lupus erythematosus presenting as post-partum chorea. D. Thomas, P. 0. Byrne and R. L. Travers, Aust. N.Z:J. Med., 1979, 9, pp. 568-570.
A case of systemic lupus erythematosus (SLE) is reported in which chorea was the dominant clinical feature, and which presented following a spontaneous mid-trimester abortion. The diagnosis, natural history and management of this uncommon manifestation of CNS lupus is discussed, as well as the influence of pregnancy on disease activity in SLE. Case Report A 22-year-old primigravida presented in March I978 at 27 weeks gestation with an inevitable abortion. Following artificial rupture of the membranes and oxytocin infusion a non-variable foetus of 20 weeks size was produced. Four weeks later she developed uncontrollable fidgety movements and dcpression. Other symptoms included an erythematous rash on the limbs and arthralgia chiefly affecting the wrists and ankles. There was no past history of rheumatic fever. ticuropsychiatric abnormality or drug ingestion. On admission to hospital she was emotionally labile with uncontrolkable periods of crying. There were bilateral choreiform movements more marked on the left side affecting the arms. legs and face. The movements were sudden, aimless and periodically violent, exacerbated by volition and ceasing durins sleep. The speech was explosive and indistinct. sessment showed mild loss of higher centres function and features of paranoid psychosis. On general examination there was a fine macular crythematous rash on the extensor surfaces of the arms and legs: in addition several bruises were present on the left arm. There w'as no facial rash or cutaneous vasculitis. There was no evidence of active synovitis, subcutaneous nodules or lymphadenopathy. The throat was reddened, without pustules. The temperature was 3 6 , 8 T , the blood pressure 120/80 mmHg, and the pulse 78 beats per minute and regular. The heart sounds were normal and there were no murmurs. Examination of the respiratory system was normal, and in the abdomen there was no enlargement o f liver or spleen. _____-.. .___ * Registrar in Rheumatology
fSHO in Medicine, Mayday Hospital. $Registrar in Rheumatology, Hammersmith Hospital. Correspondence: Dr. R. L. Travers, 33 Queens Road Melbourne, Victoria 3004 Accepted for publication: 22 March, 1979
I n w r tigutions The ESR was 66 mm in one hour, haemoglobin 12.8 g;dl. white cell count 8 . 0 IOY/il ~ (normal differential) with a normal blood film. Platelet count was 69 x 1O9.t. A S 0 titre was repeatedly normal and throat swab grew no pathogens. Latex and LE cclls tests were positive. antinuclear antibodies positive to a titre of 1 in 160 units, (speckled pattern) and antiDNA antibodies 49":;(Farr Assay normal 0.. 30",;,). Serological tests for syphilis were negative. Serum IgG and IgA were normal. and IgM 8.4 g." (normal 0.6 2.8). Scrum complement components Cl150>C2. C, were at the lower limit of normol (CH5(> 61'!,, (N50 125); C, 46:; (N40 200); -.75"k'(iS60-135)). Immune complexes were not detected by C,, binding assay. Coagulation profile was normal. Urea and electrolytes. creatinine and creatinine clearance were normal. There were no cells or casts on microscopy of fresh urine specimens. Ijrinc protein excretion was 0.15 g.24 hours. Serum copper, caeruloplasmin and thyroxine were normal. Radiology of the skull, chest and joints were normal. An ECG showed sinus rhythm with a normal PR interval. At lumbar puncture the opening pressure was 110 mm CSF, and the fluid was accllular and sterile. Cerebrospinal fluid protein was 0 . 2 g/l, with a slight excess of globulins. CSF complement components C,, C,, C, were undetected (Miinchi radial immunodiffusion technique). Electroencephalogram showed diffuse fast wave activity and a few episodes of low voltage theta waves in the temporal regions. An oxygen-15 brain scan showed widcspread abnormalities. (Pig. 1). The chorea was improved by thiopropazate (Dartalan) 15- 45 nigdaily and lasted seven weeks. Anti-DNA antibodies remained elevated throughout.
Discussion
Transient chorea is characteristic of rheumatic fever and may occur in the absence of other features.' In this patient, however, the presence of paranoid psychosis and thrombocytopenia accompanying rash and arthralgia suggested an alternative diagnosis. The positive LE cell preparations, antinuclear antibodies and raised anti-DNA antibodies confirmed SLE. Other possible causes of chorea (rheumatic fever, Wilson's disease, thyrotoxicosis) were excluded, and there was no history of previous abnormal drug use. Cerebral involvement in SLE may occur as an isolated event without any other clinical or
OCTOBER
FIGURE 1,
1979
SLE PRESENTING AS POST-PARTUM CHOREA
Oxygen-15 study of the left hemisphere using
”Or and Ci60,. Multiple defects (arrowed) are seen in the
-parietal
and occipital cortex to both metabolism
F66.P)artd flow (below). In addition there is a large basal defect (a) affecting mainly the metabolism image [Csurtesy of Dr. T. Jones, MRC Cyclotron Unit, Hammersmith Hospital)
serological evidence of disease, and may antedate other features by several years.’ A wide spectrum of neuropsychiatric abnormalities have been reported and particular difficulties in diagnosis arise when psychiatric symptoms predominate. Furthermore there is no single test or group of tests which will establish a definite diagnosis and electroencephalography, conventional brain scans and CSF examination are not consistently abnormal to be ~ s e f u 1 . jClaims .~ have been made that lowered levels of CSF complement (C,) correlate well with activity of CNS lupus.s This has not been confirmed by other workers who have also emphasised technical difficulties in C, estimation (related to C, instability in frozen samples and to low concentrations normally present in CSF)? The inability to detect CSF complement in this case is therefore of questionable diagnostic value.
569
Recently a technique for the study of cerebral metabolism and blood flow using the short-lived isotope 15-oxygen has been used in SLE patients with and without clinical cerebral involvement and a high proportion were found to have abnormalities.6 In this patient the technique showed evidence of diffuse cortical involvement as well as a large basal defect uhich may conform to the region of‘ the basal ganglia (Fig. I). The commonest autopsy finding in cases of chorea in SLE was widespread involvement of small vessels of the cerebral cortex and in only a single case was there involvement of the basal ganglia.’ A review of reported cases of chorea in SLE by Donaldson and Espiner established important features.8 In eight out of 22 cases, chorea was the initial manifestation and preceded other features by a mean duration of three years. The mean duration of chorea was eight weeks (longest was 5; months). It differs from Sydenham’s chorea in several aspects. Twenty-three of the 24 patients were female, the inean age of onset was 18 years and additional neurological abnormalities were seen in over 6004. In this review the mortality was almost 20% at six months. but subsequent isolated case reports indicate a better prognosis .3.9 The benefit of steroid therapy in the chorea of SLE is anecdotal and the place of steroid therapy in CNS lupus in general is unclear. Dubois’* has advocated the use of massive doses, whereas Sergent et ul.“ concluded that there was no evidence that very large doses are beneficial and in their series were positively detrimental. In the patient reported here, steroids were not given and therapy consisted of thiopropazate (Dartalan) which is of benefit in other forms of chorea.” The interaction of SLE and pregnancy has recently been re-evaluated. In the series of Grigor et dt3, the fertility rate of SLE patients was normal but there was a high incidence (46%) of spontaneous often mid-trimester abortions. Of pregnancies occurring prior to the onset of clinical SLE, 25”/, ended in spontaneous abortion. The effect of pregnancy upon disease activity in SLE is unpredictable. In a study of 217 SLE pregnancies, of the 158 where information was
570
THOMAS ET AL.
___
available, there was exacerbation in 37%, remission in 9Y.I and no observable effect in 507; with a 4% incidence of pre-e~lampsia.'~ Although exacerbation occurred at any time during pregnancy, the risk was seven times as great during the eight-weeks post-partum. The combination of two recognised features of SLE (chorea >as an initial manifestation and presentation in the post-partum period) seen in the case described here, has not previously been reported. Acknowledgement W e wish to thank Lk. F. G. Campbell, Consultant Neurologist at Mayday IIospital. for permission to report thc CMONIIS, J., I.I:WKONIA. R..HKI:SSIIiAh, R and Ih'GHlS. G. R. V (1978)- S)stemic lupus erythemstosus: A progpecttveanalysis. Ann. rhrmz. uis 37, 1'1 -128. Dumis. E. I.. (19%): In: L.upiis Erythemorurus: a rmicw ofthe current Status of discotd and systeinic lupus erythcmatosus and their vanants. E.. L. Duhois (ed.),2nd e d i t i o n (revised). 1.0s Angeles. 7:niversity of Southern California HEXTFI-T.
.~ . ( H . K S i l l h ,M. D., Ki I:MP'UFR, M. S. and i.lPSKY, R.A (19751: us system dlsease in systemic lupua erythematosus. d m e r . J. 't4Fd. 58. 6.44 654. 12 LYON,R L L. (19621: UrngtreatmcntofHuntingdon'schorea atrialwith thiopropwatc, Brit. nied. J. 1, 1308. 1310. ON C., , fh:
N Z J Med (1979). 9, pp 568 570
CASE REPORT
Systemic Lupus Erythematosus Presenting as Post-partum Chorea D. Thomas', P. D. Byrnet and R.
L. Travers:
From the Mayday Hospital, Thornton Heath, London, UK
SUtnmary: Systemic lupus erythematosus presenting as post-partum chorea. D. Thomas, P. 0. Byrne and R. L. Travers, Aust. N.Z:J. Med., 1979, 9, pp. 568-570.
A case of systemic lupus erythematosus (SLE) is reported in which chorea was the dominant clinical feature, and which presented following a spontaneous mid-trimester abortion. The diagnosis, natural history and management of this uncommon manifestation of CNS lupus is discussed, as well as the influence of pregnancy on disease activity in SLE. Case Report A 22-year-old primigravida presented in March I978 at 27 weeks gestation with an inevitable abortion. Following artificial rupture of the membranes and oxytocin infusion a non-variable foetus of 20 weeks size was produced. Four weeks later she developed uncontrollable fidgety movements and dcpression. Other symptoms included an erythematous rash on the limbs and arthralgia chiefly affecting the wrists and ankles. There was no past history of rheumatic fever. ticuropsychiatric abnormality or drug ingestion. On admission to hospital she was emotionally labile with uncontrolkable periods of crying. There were bilateral choreiform movements more marked on the left side affecting the arms. legs and face. The movements were sudden, aimless and periodically violent, exacerbated by volition and ceasing durins sleep. The speech was explosive and indistinct. sessment showed mild loss of higher centres function and features of paranoid psychosis. On general examination there was a fine macular crythematous rash on the extensor surfaces of the arms and legs: in addition several bruises were present on the left arm. There w'as no facial rash or cutaneous vasculitis. There was no evidence of active synovitis, subcutaneous nodules or lymphadenopathy. The throat was reddened, without pustules. The temperature was 3 6 , 8 T , the blood pressure 120/80 mmHg, and the pulse 78 beats per minute and regular. The heart sounds were normal and there were no murmurs. Examination of the respiratory system was normal, and in the abdomen there was no enlargement o f liver or spleen. _____-.. .___ * Registrar in Rheumatology
fSHO in Medicine, Mayday Hospital. $Registrar in Rheumatology, Hammersmith Hospital. Correspondence: Dr. R. L. Travers, 33 Queens Road Melbourne, Victoria 3004 Accepted for publication: 22 March, 1979
I n w r tigutions The ESR was 66 mm in one hour, haemoglobin 12.8 g;dl. white cell count 8 . 0 IOY/il ~ (normal differential) with a normal blood film. Platelet count was 69 x 1O9.t. A S 0 titre was repeatedly normal and throat swab grew no pathogens. Latex and LE cclls tests were positive. antinuclear antibodies positive to a titre of 1 in 160 units, (speckled pattern) and antiDNA antibodies 49":;(Farr Assay normal 0.. 30",;,). Serological tests for syphilis were negative. Serum IgG and IgA were normal. and IgM 8.4 g." (normal 0.6 2.8). Scrum complement components Cl150>C2. C, were at the lower limit of normol (CH5(> 61'!,, (N50 125); C, 46:; (N40 200); -.75"k'(iS60-135)). Immune complexes were not detected by C,, binding assay. Coagulation profile was normal. Urea and electrolytes. creatinine and creatinine clearance were normal. There were no cells or casts on microscopy of fresh urine specimens. Ijrinc protein excretion was 0.15 g.24 hours. Serum copper, caeruloplasmin and thyroxine were normal. Radiology of the skull, chest and joints were normal. An ECG showed sinus rhythm with a normal PR interval. At lumbar puncture the opening pressure was 110 mm CSF, and the fluid was accllular and sterile. Cerebrospinal fluid protein was 0 . 2 g/l, with a slight excess of globulins. CSF complement components C,, C,, C, were undetected (Miinchi radial immunodiffusion technique). Electroencephalogram showed diffuse fast wave activity and a few episodes of low voltage theta waves in the temporal regions. An oxygen-15 brain scan showed widcspread abnormalities. (Pig. 1). The chorea was improved by thiopropazate (Dartalan) 15- 45 nigdaily and lasted seven weeks. Anti-DNA antibodies remained elevated throughout.
Discussion
Transient chorea is characteristic of rheumatic fever and may occur in the absence of other features.' In this patient, however, the presence of paranoid psychosis and thrombocytopenia accompanying rash and arthralgia suggested an alternative diagnosis. The positive LE cell preparations, antinuclear antibodies and raised anti-DNA antibodies confirmed SLE. Other possible causes of chorea (rheumatic fever, Wilson's disease, thyrotoxicosis) were excluded, and there was no history of previous abnormal drug use. Cerebral involvement in SLE may occur as an isolated event without any other clinical or
OCTOBER
FIGURE 1,
1979
SLE PRESENTING AS POST-PARTUM CHOREA
Oxygen-15 study of the left hemisphere using
”Or and Ci60,. Multiple defects (arrowed) are seen in the
-parietal
and occipital cortex to both metabolism
F66.P)artd flow (below). In addition there is a large basal defect (a) affecting mainly the metabolism image [Csurtesy of Dr. T. Jones, MRC Cyclotron Unit, Hammersmith Hospital)
serological evidence of disease, and may antedate other features by several years.’ A wide spectrum of neuropsychiatric abnormalities have been reported and particular difficulties in diagnosis arise when psychiatric symptoms predominate. Furthermore there is no single test or group of tests which will establish a definite diagnosis and electroencephalography, conventional brain scans and CSF examination are not consistently abnormal to be ~ s e f u 1 . jClaims .~ have been made that lowered levels of CSF complement (C,) correlate well with activity of CNS lupus.s This has not been confirmed by other workers who have also emphasised technical difficulties in C, estimation (related to C, instability in frozen samples and to low concentrations normally present in CSF)? The inability to detect CSF complement in this case is therefore of questionable diagnostic value.
569
Recently a technique for the study of cerebral metabolism and blood flow using the short-lived isotope 15-oxygen has been used in SLE patients with and without clinical cerebral involvement and a high proportion were found to have abnormalities.6 In this patient the technique showed evidence of diffuse cortical involvement as well as a large basal defect uhich may conform to the region of‘ the basal ganglia (Fig. I). The commonest autopsy finding in cases of chorea in SLE was widespread involvement of small vessels of the cerebral cortex and in only a single case was there involvement of the basal ganglia.’ A review of reported cases of chorea in SLE by Donaldson and Espiner established important features.8 In eight out of 22 cases, chorea was the initial manifestation and preceded other features by a mean duration of three years. The mean duration of chorea was eight weeks (longest was 5; months). It differs from Sydenham’s chorea in several aspects. Twenty-three of the 24 patients were female, the inean age of onset was 18 years and additional neurological abnormalities were seen in over 6004. In this review the mortality was almost 20% at six months. but subsequent isolated case reports indicate a better prognosis .3.9 The benefit of steroid therapy in the chorea of SLE is anecdotal and the place of steroid therapy in CNS lupus in general is unclear. Dubois’* has advocated the use of massive doses, whereas Sergent et ul.“ concluded that there was no evidence that very large doses are beneficial and in their series were positively detrimental. In the patient reported here, steroids were not given and therapy consisted of thiopropazate (Dartalan) which is of benefit in other forms of chorea.” The interaction of SLE and pregnancy has recently been re-evaluated. In the series of Grigor et dt3, the fertility rate of SLE patients was normal but there was a high incidence (46%) of spontaneous often mid-trimester abortions. Of pregnancies occurring prior to the onset of clinical SLE, 25”/, ended in spontaneous abortion. The effect of pregnancy upon disease activity in SLE is unpredictable. In a study of 217 SLE pregnancies, of the 158 where information was
570
THOMAS ET AL.
___
available, there was exacerbation in 37%, remission in 9Y.I and no observable effect in 507; with a 4% incidence of pre-e~lampsia.'~ Although exacerbation occurred at any time during pregnancy, the risk was seven times as great during the eight-weeks post-partum. The combination of two recognised features of SLE (chorea >as an initial manifestation and presentation in the post-partum period) seen in the case described here, has not previously been reported. Acknowledgement W e wish to thank Lk. F. G. Campbell, Consultant Neurologist at Mayday IIospital. for permission to report thc CMONIIS, J., I.I:WKONIA. R..HKI:SSIIiAh, R and Ih'GHlS. G. R. V (1978)- S)stemic lupus erythemstosus: A progpecttveanalysis. Ann. rhrmz. uis 37, 1'1 -128. Dumis. E. I.. (19%): In: L.upiis Erythemorurus: a rmicw ofthe current Status of discotd and systeinic lupus erythcmatosus and their vanants. E.. L. Duhois (ed.),2nd e d i t i o n (revised). 1.0s Angeles. 7:niversity of Southern California HEXTFI-T.
.~ . ( H . K S i l l h ,M. D., Ki I:MP'UFR, M. S. and i.lPSKY, R.A (19751: us system dlsease in systemic lupua erythematosus. d m e r . J. 't4Fd. 58. 6.44 654. 12 LYON,R L L. (19621: UrngtreatmcntofHuntingdon'schorea atrialwith thiopropwatc, Brit. nied. J. 1, 1308. 1310. ON C., , fh:
